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Low affinity cationic amino acid transporter 2 Mitochondrial ornithine transporter 2 Unknown Solute carrier family 22 member 6 Alpha- aminoadipic semialdehyde synthase, mitochondrial Alpha- aminoadipic semialdehyde synthase, mitochondrial Alpha- aminoadipic semialdehyde dehydrogenase Kynurenine/alpha- aminoadipate aminotransferase, mitochondrial Enoyl-CoA hydratase, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Peroxisomal sarcosine oxidase Glutaryl-CoA dehydrogenase, mitochondrial Oxoglutarate dehydrogenase complex Acetyl-CoA L-Lysine L-Lysine L-Lysine Allysine Allysine Oxoadipic acid Oxoadipic acid NADPH Oxoglutaric acid Saccharopine NADP H2O H2O NAD L-Glutamic acid NADH NAD H2O Aminoadipic acid NADH Oxoglutaric acid L-Glutamic acid 3-Hydroxybutyryl-CoA Crotonoyl-CoA H2O NAD Acetoacetyl-CoA NADH CoA L-Pipecolic acid O2 (S)-2,3,4,5- Tetrahydropiperidine-2- carboxylate H2O2 Glutaryl-CoA CO2 CoA H2O Pyridoxal 5'-phosphate Thiamine pyrophosphate Lipoamide FAD Solute carrier family 22 member 6 Citric Acid Cycle Biotin Metabolism FAD FAD Inner Mitochondria Membrane Outer Mitochondria Membrane Mitochondrial Intermembrane Space Peroxisome Mitochondria Extracellular Space Intracellular Space Liver Extracellular Space Mitochondrial matrix Liver Hepatic Cell Symptoms of the disease include mild to severe intellectual disability, developmental delay, ataxia, muscular hypotonia, and epilepsy. However, most cases are asymptomatic.
Mitochondria SLC7A2 SLC25A2 Unknown SLC22A6 AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 Acetyl-CoA L-Lysine L-Lysine L-Lysine Allysine Allysine Oxoadipic acid Oxoadipic acid NADPH Oxoglutaric acid Saccharopine NADP Water Water NAD L-Glutamic acid NADH NAD Water Aminoadipic acid NADH Oxoglutaric acid L-Glutamic acid 3- Hydroxybutyryl- CoA Crotonoyl-CoA Water NAD Acetoacetyl-CoA NADH Coenzyme A L-Pipecolic acid Oxygen (S)-2,3,4,5- Tetrahydropiperidine- 2-carboxylate Hydrogen peroxide Glutaryl-CoA Carbon dioxide Coenzyme A Water SLC22A6 Citric Acid Cycle Biotin Metabolism